FEATURE ARTICLE
Dermatology Emergencies
Roselyn Kellen, Joshua M. Berlin
ABSTRACT: Dermatology is primarily an outpatient spe-
cialty. However, it is important to recognize certain
conditions that require referral for inpatient management
as well as for healthcare professionals to appreciate the
types of patients who need to be triaged appropriately in
the emergency room or urgent care setting. This article
will serve to summarize some of the diagnostic features
and management of these patients.
Key words: Dermatology, Emergency Healthcare,
StevensYJohnson Syndrome, Toxic Epidermal Necrolysis
D ermatology is primarily an outpatient
specialty, yet it is important to recognize
certain conditions that require referral
for inpatient management. Furthermore,
healthcare professionals in the emergency
room or urgent care settings need to appropriately triage
patients who present with dermatological findings. This
article will serve to summarize some of the diagnostic
features and management of these patients.
STEVENSYJOHNSON SYNDROME AND TOXIC
EPIDERMAL NECROLYSIS
Clinical Presentation and Diagnosis
StevensYJohnson syndrome (SJS) and toxic epidermal
necrolysis (TEN) are rare but severe adverse cutaneous
drug reactions characterized by erythema, hemorrhagic
erosions, and separation of the epidermis, often with
involvement of the mucous membranes (Harr &
French, 2010; Mockenhaupt, 2014). SJS and TEN are
best thought of as a single clinical entity that presents
on two ends of a spectrum based on body surface area
(BSA) involvement (Mockenhaupt, 2014).
Roselyn Kellen, BA, Weill Medical College, Cornell University,
New York, NY.
Joshua M. Berlin, MD, FAAD, Dermatology Associates, P.A. of
the Palm Beaches, Boynton Beach, FL.
The authors declare no conflict of interest.
Correspondence concerning this article should be addressed to
Joshua M. Berlin, MD, FAAD, Dermatology Associates, P.A. of the
Palm Beaches, 10301 Hagan Ranch Road, Suite D390, Boynton
Beach, FL 33437.
E-mail: joshberlin@hotmail.com
DOI: 10.1097/JDN.0000000000000222
VOLUME 8 | NUMBER 3 | MAY/JUNE 2016
Copyright © 2016 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.
Prodromal symptoms of SJS and TEN are vague, in-
cluding fever, malaise, headache, cough, stinging eyes, and
conjunctivitis, making early diagnosis challenging (Harr &
French, 2010; Usatine, Smith, Mayeaux, Chumley, &
Tysinger, 2009). One to three days later, cutaneous findings
appear, beginning on the trunk, face, palms, and soles
(Harr & French, 2010). In SJS, the primary lesions are
dusky red or flat atypical target lesions, usually isolated,
but lesions can become confluent on the face and trunk
(Harr & French, 2010). Systemic symptoms are usually
present, and epidermal detachment is found on G10% of
BSA (Harr & French, 2010). In SJSYTEN overlap, the
primary lesions and distribution are similar to those of
SJS; however, systemic symptoms are always present, and
epidermal detachment affects 10%Y30% of BSA (Harr &
French, 2010). In TEN, the primary lesions include poorly
demarcated erythematous plaques, and there is consider-
able confluence of lesions all over the body (Harr & French,
2010). Systemic symptoms are always present, and the
involved BSA is 930% (Harr & French, 2010). More
than 90% of patients with TEN develop erosions of the
buccal, genital, or oral mucosa, and the respiratory and
gastrointestinal tracts may be also affected (Harr &
French, 2010; Revuz et al., 1987). Skin lesions can also
include bullae and ulcerations, which can be extensive
and affect most of the BSA (Hafermann, Barber, Dreskin,
& Lindberg, 2014; Kaur & Dogra, 2013). Patients may
experience ocular disease, ranging from mild conjuncti-
val injection to corneal erosions and ulcerations (Chang
et al., 2007). Additional manifestations include hypoten-
sion, renal failure, respiratory failure, seizures, and coma
(Usatine & Sandy, 2010).
Diagnosis relies on clinical findings together with his-
tological evidence of full-thickness epidermal necrolysis
attributed to apoptosis of the keratinocytes (Harr &
French, 2010). SJS/TEN should be in the differential for
any patient showing characteristic skin lesions, and mucosal
involvement should be with high suspicion for SJS/TEN
and requires that a skin biopsy should be obtained (Harr
& French, 2010). Direct immunofluorescence (DIF) should
also be performed to rule out other autoimmune blistering
diseases (Harr & French, 2010). Laboratory findings can
include lymphopenia, neutropenia, thrombocytopenia,
elevated erythrocyte sedimentation rate, transaminases,
and blood urea nitrogen (Usatine & Sandy, 2010). The
193
Nikolsky sign, by which lateral pressure on erythematous
skin causes blisters because of epidermal detachment, is
often present but not specific for SJS or TEN (Harr &
French, 2010).
Etiology
Up to 75% of cases of SJS/TEN are instigated by drugs,
with the most common offenders being lamotrigine, ne-
virapine, allopurinol, sulfonamides, carbamazepine, phe-
nytoin, phenobarbital, and oxicam-type nonsteroidal
anti-inflammatory drugs such as meloxicam (Harr &
French, 2010; Mockenhaupt, 2014; Mockenhaupt et al.,
2008). In one case-control study, the median time be-
tween beginning a high-risk drug and index day was less
than 4 weeks (Mockenhaupt, 2014). Infections such as
mycoplasma pneumonia, influenza-like illnesses, and herpes
simplex virus have also been implicated in SJS/TEN (Harr
& French, 2010; Mockenhaupt, 2014). Associations have
also been reported with vaccinations, radiation, sunlight
exposure, pregnancy, connective tissue diseases, and neo-
plasms (Usatine & Sandy, 2010).
In addition, it has been known since the 1980s that there
is a genetic component to developing disease (Roujeau
et al., 1987). Associations have been found between
sulfonamide-induced TEN with human leukocyte anti-
gen (HLA)-A29, B12, and DR7 and oxicam-induced
TEN with HLA-A2 and B12 (Roujeau et al., 1987). Recent
studies in the Han Chinese population suggest an asso-
ciation between carbamazepine-induced SJS and HLA-
B*1502 (Chung et al., 2004).
Management
Because the exact cause of SJS/TEN is unknown, treatment
consists of cessation of likely causative agents, supportive
care, and alleviating symptoms (Harr & French, 2012).
Even after discontinuation of certain drugs, damage to
the kidneys or liver, in addition to the long half-lives and
reactive metabolites of certain drugs, can contribute to
further morbidity (Harr & French, 2012). Most patients
will require intensive care unit monitoring, with particular
attention to fluids and electrolytes, nutritional require-
ments, wound care, and monitoring for infections. Blisters
should not be debrided because their presence enhances
reepithelialization (Harr & French, 2010). Erosions can be
treated with chlorhexidine, octenisept, or polyhexanide and
covered with nonadherent gauze; topical sulfa medications
should not be used (Harr & French, 2010; Mockenhaupt,
2014). Ophthalmologic consult is necessary to prevent
permanent ocular sequelae (Mockenhaupt, 2014).
Average mortality rates are 1%Y5% in SJS and
25%Y35% in TEN, with higher rates of death in elderly
patients and those with extensive BSA involvement (Harr
& French, 2010). The mortality rate of patients with TEN
can be estimated using SCORTEN, a severity-of-illness
score that takes into account seven independent risk factors
for death: age, an underlying malignancy, tachycardia, BSA
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Copyright © 2016 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.
involvement, and serum urea, glucose, and bicarbonate
values (Bastuji-Garin et al., 2000).
Although there are no clinical trials to support a spe-
cific therapy for SJS/TEN, several systemic drugs have been
used in the literature such as systemic steroids, cyclospor-
ine, and intravenous immunoglobulin (IVIG) to benefit
patients (Harr & French, 2010; Law & Leung, 2015). An
open, Phase II trial in France involving cyclosporine for
SJS and TEN revealed lower death rates compared with
those estimated using SCORTEN scores (Valeyrie-Allanore
et al., 2010). The data regarding the use of IVIG are con-
flicting, with some studies showing no advantage and
others reporting improvement (Chen, Wang, Zeng, &
Xu, 2010; Mittmann, Chan, Knowles, Cosentino, & Shear,
2006; Stella, Clemente, Bollero, Risso, & Dalmasso, 2007).
In addition, there have been several case reports describing
the successful use of infliximab, plasmapheresis, hyperbaric
oxygen, and cyclophosphamide for TEN (Harr & French,
2010; Mockenhaupt, 2014).
Although the skin usually heals, more than half of
survivors of TEN are left with residual complications in-
cluding changes to the skin, hair, nails, mucous membranes,
eyes, and respiratory epithelium (Harr & French, 2010,
2012; Mockenhaupt, 2014). Skin changes include hypo-
pigmentation or hyperpigmentation, pruritus, xerosis, and
hyperhidrosis (Mockenhaupt, 2014). Hair and nail changes
include reversible hair loss, onycholysis, and onychodys-
trophy (Mockenhaupt, 2014). In one study, 73% of pa-
tients with TEN with mucosal involvement had permanent
mucosal sequelae (Oplatek et al., 2006). These mucosal
changes most often affect the oral and esophageal mucosa
(loss of papilla on the tongue, impaired taste, strictures of
the esophagus) but can also affect the genital mucosa
(adhesions in the urethra, anus, and vagina; Mockenhaupt,
2014; Oplatek et al., 2006). Ocular complications include
severe dry eyes, trichiasis, symblepharon, distichiasis,
ocular scarring, and blindness (Usatine & Sandy, 2010;
Yip et al., 2007). The most common cause of death from
TEN is septicemia, often because of central venous lines
(Mockenhaupt, 2014). One of the most serious com-
plications of TEN is damage to the tracheal and bron-
chial epithelium, which occurs in up to 20% of patients
(Mockenhaupt, 2014).
NECROTIZING FASCIITIS
Clinical Presentation and Diagnosis
Necrotizing fasciitis (NF) is a rapidly progressive necrosis
of skin, muscle, and soft tissue that spreads along fascia
planes at a rate of 2Y3 cm per hour (Misiakos et al., 2014).
The areas most often affected are the lower extremities,
abdomen, and perineum, with the latter referred to as
Fournier gangrene (Usatine & Sandy, 2010).
Early signs include the triad of erythema, swelling,
and tenderness, along with fever and chills (Usatine &
Sandy, 2010). Anaerobic bacteria can produce gas in the
Journal of the Dermatology Nurses’ Association
tissue that may be felt as crepitus upon palpation. Even-
tually, nerve destruction can lead to motor and sensory
deficits (Morgan, 2010). A classic finding is increasingly
severe pain out of proportion to the physical examination,
because of hypoxia and swelling of the tissue, which can
help rule out a differential of cellulitis (Morgan, 2010;
Usatine & Sandy, 2010). The erythematous skin then
develops a dusky blue hue with yellow vesicular and bullous
lesions, accompanied by serosanguineous drainage (Usatine
& Sandy, 2010). Four to five days later, violaceous bullae
are seen, along with gangrenous skin and a ‘‘woody’’ feel
to the tissue (James, Berger, Elston, & Odom, 2006;
Misiakos et al., 2014; Usatine & Sandy, 2010). After about
10 days, eschar sloughs off (Usatine & Sandy, 2010).
Those with fulminant disease present with severe septic
shock and multiple organ dysfunction syndrome and rapidly
deteriorate within hours (Misiakos et al., 2014; Park, Jung,
Jung, Shin, & Hwang, 2009).
NF is often diagnosed clinically, although definitive diag-
nosis is surgical, showing ‘‘dishwater fluid’’ compromised
of necrotic tissue and neutrophils (Misiakos et al., 2014).
Suspected cases require gram staining of the drainage as
well as histological examination and culture of deep tissue
biopsies (Usatine & Sandy, 2010). Laboratory findings,
although not specific, include white blood cell (WBC)
count 9 14,000 cells per mm3, serum sodium G 135 mEq/L,
and blood urea nitrogen 915 mg/dl (Usatine & Sandy, 2010).
X-rays can show evidence of gas in the soft tissue, but
computed tomography and magnetic resonance imaging
can show with better detail the extent of infection, swell-
ing, inflammation, and presence of gas (Misiakos et al.,
2014; Morgan, 2010).
Wong, Khin, Heng, Tan, and Low (2004) created a scor-
ing system known as LRINEC (Laboratory Risk Indicator
for Necrotizing Fasciitis) to distinguish between necrotizing
and nonnecrotizing soft tissue infections based on laboratory
values (Wong et al., 2004). They identified six variables
associated with necrotizing infections: C-reactive protein,
WBC count, hemoglobin, serum sodium, creatinine, and
serum glucose levels. Using this scoring system, intermediate-
and high-risk patients (scoring 96/13) had a positive pre-
dictive value of 92% and negative predictive value of 96%.
Pathophysiology
Although many cases of NF are idiopathic, patients should
be asked about a history of trauma including insect bites,
recent surgery, skin infection, and illicit intravenous drug
use (Morgan, 2010).
Type I NF accounts for 70%Y80% of cases and tends
to affect patients with recent trauma or surgery, those who
are immunocompromised, and patients with underly-
ing abdominal disease (Morgan, 2010; Usatine & Sandy,
2010). It is a polymicrobial infection with the most common
offenders being non-group-A Streptococci, Bacteroides,
Enterobacteriaceae, and Peptostreptococcus (Usatine et al.,
2009). Type II NF, responsible for 20%Y30% of cases, is
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because of infection by group A "-hemolytic strep (Strep-
tococcus pyogenes) and less commonly by Staphylocccus
(S.) aureus (Misiakos et al., 2014; Morgan, 2010). In
Asia, NF can be associated with certain types of raw or
undercooked seafood containing Vibrio spp., Aeromonas
spp., and Shewanella spp. (Park et al., 2009). The rate of
progression depends on the microorganisms: Type 2 NF
spreads at a much faster rate than Type 1 NF (Morgan,
2010). The resulting tissue necrosis is because of damage
from bacterial enzymes but also thrombosis of blood vessels
in the hypodermis (Park et al., 2009).
Management
Early detection is imperative so that patients can undergo
surgical debridement and receive broad-spectrum antibi-
otics, with the former being the most crucial component
of treatment (Misiakos et al., 2014).
Early and aggressive surgical interventions are crucial;
in one study, surgical delay of 24 hours increased the mor-
tality rate of Vibrio spp.-induced NF from 35% to 53%
(Klontz et al., 1988). Even a 12-hour delay in surgery
can be fatal in patients with fulminant disease (Misiakos
et al., 2014). Surgical management requires debridement,
necrosectomy, and fasciotomy (Misiakos et al., 2014). After
surgery, fluid losses, nutritional needs, and wound healing
must be carefully monitored. The use of vacuum-assisted
closure dressings aids in wound cleaning and enhances the
formation of granulation tissue (Misiakos et al., 2014).
Empiric antibiotics must cover gram positives (e.g.,
vancomycin, linezolid, ampicillin plus gentamycin), gram
negatives (e.g., quinolones), and anaerobes (e.g., clinda-
mycin or metronidazole; Usatine & Sandy, 2010). An in
vitro study found that clindamycin, either alone or with
a penicillin, decreased early release of streptococcal exo-
toxin A compared with using a penicillin alone (Coyle,
Cha, & Rybak, 2003).
For Type 1 NF, ampicillin or ampicillinYsulbactam in
combination with metronidazole or clindamycin can be
used (Misiakos et al., 2014). For Type 2 NF, antibiotics
must cover S. pyogenes and S. aureus, which often coexist,
and can include first- or second-generation cephalo-
sporines (for methicillin-resistant S. aureus [MRSA]), van-
comycin, daptomycin, or linezolid (Misiakos et al., 2014).
Antibiotics can be tailored based on blood, wound, and
tissue cultures but must be continued for at least 48 hours
after patients are clinically and hemodynamically stabilized
(Misiakos et al., 2014). Patients often require antibiotics
for 4Y6 weeks (Misiakos et al., 2014). There may be a role
for adjunct hyperbaric oxygen and IVIG (especially for
streptococcal toxic shock syndrome [TSS]), but further
studies with these agents are required (Kaul et al., 1999;
Krenk, Nielsen, & Christensen, 2007). Hyperbaric oxy-
gen appears to reduce mortality and amputation rates for
Clostridium spp.-associated NF by reducing "-toxin pro-
duction and enhancing neutrophil activity (Escobar, Slade,
Hunt, & Cianci, 2005; Morgan, 2010).
195
 Risk factors associated with a poor prognosis are dia-
betes mellitus (the most common associated morbidity),
chronic renal failure, liver cirrhosis, chronic alcohol abuse,
and immunosuppression (Misiakos et al., 2014). Some
patients require amputation of limbs if there is extensive
necrosis or they are not able to tolerate the lengthy op-
eration needed to save the limb (Misiakos et al., 2014;
Tang, Ho, Fung, Yuen, & Leong, 2001). Although ampu-
tations do not reduce mortality, patients have fewer repeat
operations (Wong et al., 2003). The median mortality
rate for NF is 32.3%, which increases to 70% in patients
with sepsis and to almost 100% without treatment
(Misiakos et al., 2014; Usatine & Sandy, 2010).
ROCKY MOUNTAIN SPOTTED FEVER
Clinical Presentation and Diagnosis
The classic triad of Rocky Mountain spotted fever (RMSF)
is fever, rash, and severe headache, although myalgias
(especially involving the abdominal, back, and calf
muscles), nausea, vomiting, abdominal pain (especially in
children, which can be mistaken for appendicitis), conjunc-
tival injection, and altered mental status can also be present
(Cunha, 2008; Salinas, Greenfield, Little, & Voskuhl, 2010;
Usatine & Sandy, 2010). Less commonly seen are manifes-
tations such as periorbital edema, edema of the dorsum of
the hands and feet, and hepatosplenomegaly (Cunha, 2008).
The characteristic rash of RSMF, which appears
several days after the fever, starts on the wrists and ankles
before spreading to the palms, soles, trunk, and extrem-
ities, often sparing the face (Salinas et al., 2010; Usatine
& Sandy, 2010; Wolff & Fitzpatrick, 2005). It typically
evolves from small, blanching, nonpruritic macules to
maculopapular (Days 1Y2) and finally petechial lesions
(Days 3Y5; Salinas et al., 2010; Usatine & Sandy, 2010).
The petechial lesions often coalesce to form ecchymoses,
resulting in a spotted appearance of the skin (Usatine
et al., 2009). It is important not to miss the early pale,
pink papules, especially in dark-skinned patients (Cunha,
2008). It is also possible to see skin desquamation as the
disease progresses (Usatine et al., 2009).
The diagnosis is based on clinical presentation with a
history favoring exposure to a tick (Salinas et al., 2010).
It is helpful to ask patients about exposure to environ-
ments where ticks are found because the tick bite itself is
painless and often goes unnoticed. Laboratory findings
may show thrombocytopenia, hyponatremia, and elevated
transaminases with a normal WBC count (Usatine &
Sandy, 2010). Chest imaging can reveal interstitial pneu-
monitis or infiltrates because of myocarditis (Cunha, 2008;
Salinas et al., 2010). Serum titers of anti-Rickettsia (R.)
rickettsi antibodies with greater than a fourfold increase
are confirmatory, but this may take up to 10 days after the
onset of disease (Salinas et al., 2010; Wolff & Fitzpatrick,
2005). Punch biopsy with DIF may reveal rickettsial orga-
nisms in the endothelium of blood vessels (Salinas et al.,
196
Copyright © 2016 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.
2010; Usatine & Sandy, 2010). Although polymerase
chain reaction of skin biopsies alone is not sensitive, this
method is often combined with immunohistochemical
staining (Chapman et al., 2006).
Etiology
RMSF is caused by the gram-negative intracellular bac-
terium R. rickettsi. Although several ticks can transmit
this disease, the two most common are the wood tick (Der-
macentor andersoni) in the western United States and the
brown dog tick (Dermacentor variabilis) in the eastern
United States (Chapman et al., 2006; Usatine & Sandy,
2010). After the tick bites, the bacterium travels through
the lymphatic system and multiplies in endothelial cells
causing vascular inflammation that presents as a vascu-
litis (Salinas et al., 2010). Although any organ can be
involved, the skin and adrenals glands are most com-
monly affected (Usatine & Sandy, 2010).
Management
Doxycycline 100 mg twice daily (orally or intravenously),
or 2.2 mg/kg for children G 100 lbs, is the first-line treat-
ment, with tetracycline being an alternative agent (Chapman
et al., 2006). The Centers for Disease Control and Pre-
vention recommends antibiotics for 3 days after the fever
disappears and until the patient shows clinical improve-
ment, which is usually 5Y7 days (Chapman et al., 2006). In
pregnancy, chloramphenicol is recommended (Yu, Merigan,
& Barriere, 1999).
With a case fatality rate of 5%Y10%, RMSF is the most
commonly fatal rickettsial disease in the United States
(Chapman et al., 2006; Salinas et al., 2010; Usatine &
Sandy, 2010). The severity varies greatly with some pa-
tients being stable enough to be treated on an outpatient
basis and others requiring hospitalization (Usatine &
Sandy, 2010). Factors associated with more severe disease
include older age, male, black race, chronic alcoholism,
and glucose-6-phosphate-dehydrogenase deficiency
(Walker & Raoult, 2005). Aside from severe cases,
persistent fever after 48 hours of antibiotics should raise
concerns regarding the diagnosis (Salinas et al., 2010).
Complications of RMSF include gangrene of the digits
sometimes requiring amputation, myocarditis, meningo-
encephalitis, meningitis, sepsis, cardiac and renal failure,
hearing loss, blindness, and other neurological deficits
(Archibald & Sexton, 1995; Salinas et al., 2010; Usatine
& Sandy, 2010). It is imperative for healthcare pro-
fessionals to report cases of RMSF to the state health
department (Usatine & Sandy, 2010).
STAPHYLOCOCCAL AND STREPTOCOCCAL TSS
Clinical Presentation and Diagnosis
TSS, first described by Todd, Fishaut, Kapral, and Welch
in 1978, presents with sudden-onset high fever, rash,
hypotension, and multisystem organ dysfunction over the
course of several hours, often in young, healthy patients
Journal of the Dermatology Nurses’ Association
(Andrews, Parent, Barry, & Parsonnet, 2001; Silversides,
Lappin, & Ferguson, 2010). A prodromal influenza-like
illness usually occurs 1Y2 days before patients present for
medical advice (Silversides et al., 2010). The rash of TSS
is often described as a sunburn-like rash, macular and
erythematous, and can be widespread or localized (Reiss,
2000; Silversides et al., 2010). Mucous membrane in-
volvement may include oropharyngeal hyperemia, straw-
berry tongue, and nonpurulent conjunctivitis (Reiss, 2000).
Patients may display tachycardia, tachypnea, dizziness,
confusion, or impaired consciousness (Silversides et al.,
2010). Other manifestations may include vomiting, watery
diarrhea, chills, and myalgia (Reiss, 2000). Approximately
1Y2 weeks into the disease course, mild desquamation occurs
over the face, trunk, and extremities, followed by full-thickness
desquamation over the palms and soles (Reiss, 2000).
Although commonly associated with tampon use, TSS
has also been reported with barrier contraceptives, in-
trauterine devices, respiratory infections, nasal packs, and
various soft tissue infections including surgical wounds,
infected burns, postpartum infections, and deep abscesses
(Ferguson & Todd, 1990; Schwartz et al., 1989). Public
education regarding the risk of TSS from tampon use has
led to a decrease in menstrual-associated TSS, whereas the
frequency of nonmenstrual TSS cases has remained rela-
tively constant (Reiss, 2000). Menstrual and nonmenstrual
cases, which appear the same clinically, now occur with
almost the same frequency (Andrews et al., 2001; Reingold,
Hargrett, Dan, et al., 1982). The former is classically seen
in White women of childbearing age, whereas the latter is
seen equally in men and women (Reiss, 2000).
Diagnosis can be challenging when patients have other
comorbidities. There are several case definitions from the
Centers for Disease Control and Prevention, but many
times, the diagnosis rests upon clinical presentation, ab-
normal laboratory values, and culture results (Reingold,
Hargrett, Shands, et al., 1982; Silversides et al., 2010).
Patients with risk factors (postoperative patients, men-
struating women) should be viewed with a high level of
suspicion (Silversides et al., 2010). In all suspected cases,
it is imperative to obtain cultures and gram staining of
potential sites of infection (Silversides et al., 2010). Blood
cultures tend to be positive in streptococcal TSS but not
staphylococcus TSS (Silversides et al., 2010). Chest im-
aging may show evidence of acute respiratory distress
syndrome (ARDS) and be helpful in excluding alternative
diagnoses (Silversides et al., 2010). Polymerase-chain-
reaction-based tests for the superantigen genes, anti-TSS-
toxin-1 (TSST-1) antibody assays, and flow cytometry for
T-cell analysis may also be helpful (Ferry et al., 2008;
Granger et al., 2010; Javid Khojasteh, Rogan, Edwards-
Jones, & Foster, 2003).
Etiology
Menstrual-associated TSS is caused by S. aureus, whereas
nonmenstrual TSS is attributable to both S. aureus and
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Streptococcus pyogenes (Reiss, 2000). Both bacteria are
part of the normal flora of the skin and mucous mem-
branes; however, certain strains produce toxins that gen-
erate an overwhelming immune response resulting in a
cytokine storm (Andrews et al., 2001; Silversides et al.,
2010). Staphylococcal TSST-1 and enterotoxins and strep-
tococcal exotoxins are ‘‘superantigens’’ that activate exces-
sive numbers of T cells and enhance the release of
proinflammatory cytokines such as interleukin (IL)-2,
tumor necrosis factor ", IL-6, IL-12, and interferon +, re-
sulting in the clinical signs and symptoms of TSS (Andrews
et al., 2001; Fast, Schlievert, & Nelson, 1989; Parsonnet
& Gillis, 1988; Parsonnet, Gillis, & Pier, 1986; Reiss, 2000;
Silversides et al., 2010). Most adults have antibodies to
TSST-1; disease occurs only when a person lacks the neu-
tralizing antibody, suggesting that intrinsic host factors
play an important role in the development of disease (Reiss,
2000; Silversides et al., 2010).
Management
With a mortality rate of 4%Y22% for staphylococcal TSS
and up to 85% for streptococcal TSS, early identification
and treatment are paramount (Madhusudhan, Sambamurthy,
Williams, & Smith, 2007; Silversides et al., 2010). The
three components of managing TSS include identifying the
source of infection, providing supportive care, and admin-
istering antibiotics.
A thorough physical examination is necessary to look
for and remove foreign bodies that might be instigating the
infection, to debride infected wounds, or to drain abscesses
(Reiss, 2000). The main priority for patients with TSS
is supportive care similar to that for septic shock, which
usually warrants admission to the intensive care unit
(Silversides et al., 2010). Most patients require massive
fluid resuscitation and vasopressors because of resistant
hypotension and sometimes intubation and ventilation
(Reiss, 2000; Silversides et al., 2010). Other supportive
measures include hydrocortisone, glycemic control, blood
products, and parenteral nutrition (Silversides et al., 2010).
The third component of treatment is antibiotic therapy,
which ideally should be started after blood cultures are
taken (Silversides et al., 2010). For staphylococcal TSS,
nafcillin, cloxacillin, or flucloxacillin are used alone or
with an aminoglycoside, whereas vancomycin is reserved
for MRSA strains (Silversides et al., 2010). For strepto-
coccal TSS, clindamycin is used together with penicillin
(Madhusudhan et al., 2007).
Interestingly, antibiotics are not used to shorten the
duration of disease but rather to reduce the risk of re-
currence; up to one third of patients with menstrual TSS
who are not treated with antibiotics will experience re-
current disease (Davis, Chesney, Wand, & LaVenture,
1980; Davis et al., 1982). Those at risk are likely col-
onized with a virulent strain of S. aureus and lack the
neutralizing antibody that normally protects adults from
the toxin (Andrews et al., 2001). Patients treated with
197
antistaphylococcal antibiotics appear to decrease their risk
of recurrent disease (Andrews et al., 2001; Davis et al.,
1980, 1982). Recurrent nonmenstrual TSS is far less com-
mon, although cases have been reported (Andrews et al.,
2001). Further evaluation is required to determine the
appropriate duration of therapy to eliminate toxigenic
carriage, but previous studies suggest that a 2-week course
is sufficient (Andrews et al., 2001). IVIG might play a
role for patients with severe streptococcal disease, but
there are insufficient data to recommend a particular dosing
regimen (Darenberg et al., 2003; Schlievert, 2001;
Silversides et al., 2010).
Complications of TSS include coagulation abnormal-
ities such as disseminated intravascular coagulation, sepsis,
acute tubular necrosis, acute kidney injury, ARDS, hepatic
dysfunction, splitting of the nails, reversible hair and nail
loss, cardiac dysfunction, and central nervous system com-
plications (Reiss, 2000; Silversides et al., 2010).
CHILD ABUSE
Most children experiencing physical abuse have cutaneous
findings. We will briefly review dermatological findings
of the more commonly seen abuse patterns and mention
several cultural practices that are often mistaken for signs
of child abuse.
Bruises are a very common finding in children, es-
pecially over the knees, anterior tibia, and bony prominences
(Carpenter, 1999; Chadwick, 1992). Bruises of varying
ages in uncommon areas, such as the upper arms, medial
and posterior thigh, hands, trunk, cheeks, ears, neck, gen-
italia, and buttocks, should raise suspicion (Ermertcan &
Ertan, 2010). Bruises in infants who are not yet mobile,
especially under 9 months old, are a red flag (Labbé &
Caouette, 2001). The shape of the bruise can sometimes
give an indication to the type of object used to harm the
child (Ermertcan & Ertan, 2010; Kos & Shwayder, 2006).
Ecchymoses, abrasions, or lesions in an oval or el-
liptical pattern should raise suspicion for a bite mark, with
human bite marks typically more superficial than animal
bites (Ermertcan & Ertan, 2010). All bite marks should
prompt a full skin examination for other signs of abuse;
they can pose a concern regarding infection (Kos &
Shwayder, 2006). The shape, color, and diameter must
be recorded; photographs should be taken; and the bite
should be swabbed with a sterile cotton swab and sent to
a forensic laboratory (Ermertcan & Ertan, 2010; Kini &
Lazoritz, 1998).
Burn abuse commonly affects children under 3 years old
and can be caused by flames, cigarettes, electrical/chemical
burns, and exposure to household appliances (Ermertcan
& Ertan, 2010). Cigarette burns typically present as well-
demarcated, 7- to 10-mm, circular lesions with a deep crater,
often grouped over the face, hands, and feet (Ermertcan &
Ertan, 2010). Children forcibly lowered into hot bathwater
have symmetrical burns over the buttocks, perineum, and
lower extremities with uniform depth and well-demarcated
198
Copyright © 2016 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.
borders, often referred to as tidemarks (Stratman &
Melski, 2002; Yeoh, Nixon, Dickson, Kemp, & Sibert,
1994). Stocking and glove burns result from forced im-
mersion of the hands and feet (Ermertcan & Ertan, 2010;
Stratman & Melski, 2002). Children submerged in hot
water tend to have sparing of the flexural creases, result-
ing in zebra stripes (Ermertcan & Ertan, 2010).
The oral cavity is frequently injured in child abuse victims
so a thorough examination is necessary. Injuries can in-
clude contusions, burns, and lacerations of the tongue,
lips, buccal mucosa, palate, and gums. Children can ex-
perience fractured or displaced teeth, facial bone and jaw
fractures, and tears of the frenulum or labia (Ermertcan &
Ertan, 2010). A torn frenulum is almost always patho-
gnomonic of abuse (Kos & Shwayder, 2006). Erythema
or petechiae of the palate, especially the junction of the
soft and hard palate, is sometimes suggestive of oral sexual
abuse (Ermertcan & Ertan, 2010).
Several other findings are also concerning for child abuse.
Traumatic alopecia may be accompanied by petechiae at
the pulled hair root or a boggy scalp because of subgaleal
hemorrhage (Ermertcan & Ertan, 2010; Kos & Shwayder,
2006). The differential diagnosis includes tinea capitis,
traction alopecia, trichotillomania, loose anagen syn-
drome, and alopecia areata (Ermertcan & Ertan, 2010).
Findings of sexual abuse include abrasions and lacera-
tions of the genitalia and bite marks on the inner thighs
of genitalia (Ermertcan & Ertan, 2010). Finally, children
can present with signs of neglect such as severe dermatitis,
subcutaneous wasting, and scaly skin because of malnu-
trition and poor hygiene (Ermertcan & Ertan, 2010).
There are several cultural practices that are important
for us to be aware of because their manifestations can
mimic those of child abuse (Ermertcan & Ertan, 2010).
Cao gio, also known as coin rubbing or coining, is a
well-known practice in Southeast Asia whereby a coin is
rubbed repeatedly over pre-oiled or lubricated skin to
treat many different illnesses (Yeatman & Dang, 1980).
It can result in linear erythematous marks, petechiae, pur-
pura, and burns, most often on the back, neck, head,
shoulders, and chest (Davis, 2000; Ermertcan & Ertan,
2010; Yeatman & Dang, 1980). A practice known as
cupping creates areas of lower air pressure and suction
next to the skin, resulting in circular ecchymoses, hyper-
pigmentation, hematomas, and lacerations (Ravanfar &
Dinulos, 2010). Oils are often placed on the skin, to which
children can develop a contact dermatitis with erythema,
blisters, and scaling (Ravanfar & Dinulos, 2010). Moxi-
bustion, a component of traditional Chinese medicine,
burns herbs that are placed on the skin via acupuncture
needles, either directly or via a moxa stick, sometimes re-
sulting in burns and scars (Ravanfar & Dinulos, 2010).
Salting is a practice in Turkey whereby neonates are scrubbed
with salt for an hour, which can lead to epidermolysis,
severe hypernatremia, and scalded skin (Ravanfar &
Dinulos, 2010). Gridding, originating from Russian culture,
Journal of the Dermatology Nurses’ Association
consists of painting a grid-like pattern on the back of a
child with iodine to help with respiratory illnesses (Ravanfar
& Dinulos, 2010).
THE ERYTHRODERMIC PATIENT: MANAGEMENT
APPROACH
Clinical Presentation and Diagnosis
Erythroderma, also known as exfoliative dermatitis, is a
dermatological emergency most commonly seen in pa-
tients 41Y61 years old, with a male-to-female ratio of
2:1Y4:1 (Bruno & Grewal, 2009; Rothe, Bernstein, &
Grant-Kels, 2005). It is characterized by diffuse erythema
and scaling of the skin, often affecting more than 90% of
BSA, but sometimes sparing the nose and paranasal
areas (Okoduwa et al., 2009). Cutaneous findings often
begin as erythematous patches that grow in size, coalesce,
and spread over the body (Okoduwa et al., 2009). White
and yellow scales develop, and the skin eventually ap-
pears bright red, dry, scaly, and warm upon touch (Okoduwa
et al., 2009). Chronic erythroderma is often characterized by
lichenfication, diffuse alopecia, nail dystrophy, keratoderma,
and ectropion (Rothe et al., 2005). Nail changes include
thick, dry, and brittle nails; nail shedding; subungual hyper-
keratosis; distal onycholysis; and ridging of the nail plate
(Okoduwa et al., 2009). Patients can also report fever,
malaise, fatigue, pruritus, gynecomastia, and peripheral or
periorbital edema (Okoduwa et al., 2009; Rothe et al., 2005).
Etiology
The most common causes are psoriasis, spongiotic derma-
titis, drug eruptions, and cutaneous T-cell lymphoma (Rothe
et al., 2005). However, etiologies include exacerbation of
underlying dermatoses (psoriasis, atopic dermatitis, seb-
orrheic dermatitis, pityriasis rubra pilaris), infections, sys-
temic diseases, hematologic diseases (cutaneous T-cell
lymphoma), and solid tumor malignancies (Rothe et al.,
2005). In many cases, no underlying cause can be found,
and the condition is labeled as idiopathic. In one study
analyzing 64 cases of erythroderma, the two most com-
mon causes were underlying dermatoses (58%) and drugs
(16%), with 16% of cases labeled as idiopathic (Eugster,
Kissling, & Brand, 2001). The most common drug triggers
include antiepileptics, antibiotics, antihypertensives, cal-
cium channel blockers, cimetidine, lithium, and other topical
agents (Akhyani, Ghodsi, Toosi, & Dabbaghian, 2005;
Okoduwa et al., 2009). As the etiologies are numerous,
here, we will focus on a general approach for the man-
agement of erythrodermic patients, which is common to
all etiologies.
Management
The first step in the management of erythroderma is ob-
taining a detailed history, paying particular attention to
all systemic and topical medications. Vital signs must be
taken to ensure that patients are stable and do not require
VOLUME 8 | NUMBER 3 | MAY/JUNE 2016
Copyright © 2016 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.
immediate admission to hospital (Rothe et al., 2005).
Next, patients need a thorough skin examination, with
special focus on the nails, mucous membranes, and lymph
nodes and the presence or absence of hepatosplenomegaly
(Rothe et al., 2005). Certain physical findings can sug-
gest the underlying etiology, for example, psoriatic eryth-
roderma (psoriasiform plaques), pityriasis rubra pilaris
(islands of spared skin, orange palmoplantar keratoderma,
hyperkeratotic follicular plaques on extensor surfaces),
lichen planus (violaceous papules and buccal mucosal
lesions), erythrodermic dermatomyositis (Gottron’s papules,
heliotrope rash, periungual telangiectasias, poikiloderma),
immunobullous diseases (blisters and erosions), Sézary
syndrome (severe pruritus, lagophthalmos, alopecia, lymph-
adenopathy, hepatosplenomegaly, fissured keratoderma,
onychodystrophy, and leonine facies from skin infiltra-
tion), and scabies (burrows, especially along the flexural
wrist surfaces; Okoduwa et al., 2009; Rothe et al., 2005;
Yamashita, Abbade, Marques, & Marques, 2012).
Laboratory changes, although often not specific, in-
clude leukocytosis with eosinophilia, anemia, decreased
albumin, elevated uric acid, erythrocyte sedimentation rate,
and immunoglobulin E (Okoduwa et al., 2009; Rothe
et al., 2005; Yamashita et al., 2012). Sézary cell count
9 20% of circulating lymphocytes and a CD4YCD8 ratio
9 10 are suspicious for Sézary syndrome (Yamashita et al.,
2012). Biopsies are often nonspecific, illustrating hyperker-
atosis, parakeratosis, acanthosis, and perivascular inflam-
matory infiltrates (Okoduwa et al., 2009). Retrospective
studies show that the correlation between pathological
diagnosis and clinical diagnosis ranges from 48% to 66%;
multiple punch biopsies are usually required to establish a
diagnosis (Rothe et al., 2005; Walsh et al., 1994; Zip,
Murray, & Walsh, 1993). The use of special stains, gene
rearrangement tests (to rule out lymphoproliferative dis-
ease), and DIF (helpful for ruling out autoimmune blis-
tering diseases) can also facilitate diagnosis (Rothe et al.,
2005). Patch testing can help rule out allergic contact
dermatitis, and biopsies of abnormal lymph nodes may
reveal underlying malignancies (Rothe et al., 2005). If no
etiology is found, a systemic disease should be consid-
ered, and the patient should be surveilled appropriately
(Rothe et al., 2005).
Patients should stop taking all unnecessary medications,
especially those that are known to be possible triggers.
They often require supportive care with focus on fluid and
electrolyte requirements, nutrition, and wound care (Rothe
et al., 2005). Protein loss from scaling of the skin requires
an increase in daily protein by 25%Y30% in psoriatic
erythroderma and 10%Y15% for other causes; deficits cause
edema, muscle wasting, and hypoalbuminemia (Kanthraj
et al., 1999). For weeping or crusted lesions, emollients
and low-potency topical corticosteroids can be applied,
covered by wet dressings or the use of oatmeal baths
(Rothe et al., 2005). Caution is advised with topical immu-
nomodulators such as tacrolimus as one patient with
199
generalized leukemic erythroderma developed elevated
blood levels of tacrolimus, posing a risk for nephrotoxicity
(Teshima et al., 2003). Other supportive measures include
the use of air humidifiers to help moisturize the skin and
prevent hypothermia and oral antihistamines to relieve
pruritus (Chang et al., 2007; Rothe et al., 2005). Systemic
treatment options include corticosteroids, methotrexate,
cyclosporine, mycophenolate mofetil, and acitretin (Rothe
et al., 2005). Patients with signs of secondary infection
require systemic antibiotics, and those with edema may
benefit from a diuretic (Rothe et al., 2005).
Patients are at high risk for thermoregulatory dysfunc-
tion, fluid and electrolyte imbalances, high-output cardiac
failure, ARDS, secondary infection, and sepsis (Rothe et al.,
2005). The most common causes of death are pneumonia,
septicemia, and heart failure (Okoduwa et al., 2009).
Although drug-induced erythroderma will resolve after
cessation of the offending drug, the prognosis for other cases
of generalized erythema depends on the underlying cause.
DRUG REACTION WITH EOSINOPHILIA AND
SYSTEMIC SYMPTOMS SYNDROME
Clinical Presentation and Diagnosis
Drug reaction with eosinophilia and systemic symptoms
(DRESS) syndrome is a severe drug reaction that typically
occurs 2Y8 weeks after exposure to the causative agent
and can present with a variety of features (Choudhary,
McLeod, Torchia, & Romanelli, 2013). Certain patterns
are more commonly associated with specific drugs. How-
ever, most patients have fever, cutaneous manifestations,
and systemic findings such as lymphadenopathy, leuko-
cytosis with eosinophilia, and abnormal liver function tests
(Choudhary et al., 2013).
In one study analyzing retrospective data from 216 pa-
tients with cutaneous drug reactions and systemic symp-
toms, 73%Y100% had cutaneous findings, with the most
common patterns being a diffuse maculopapular rash and
erythroderma (Peyrière et al., 2006). However, lichenoid
dermatitis, urticaria, vesicles, bullae, pustules, purpura,
erythema multiforme lesions, facial edema, and cheilitis
can also be present (Chiou et al., 2008; Choudhary et al.,
2013; Peyrière et al., 2006). Other systemic symptoms in-
clude hematological abnormalities such as anemia, throm-
bocytopenia, and neutropenia; gastrointestinal symptoms;
renal dysfunction (especially when associated with allopu-
rinol); lung involvement (most commonly associated with
minocycline); and heart abnormalities (also commonly
associated with minocycline; Peyrière et al., 2006).
DRESS is a clinical diagnosis for which various diag-
nostic criteria have been created to try to standardize diag-
nosis (Choudhary et al., 2013). Santiago, Gonçalo, Vieira,
Coelho, and Figueiredo, (2010) reported on the benefits
of patch testing as a method to identify the culprit drug,
but this was limited to DRESS induced by antiepileptics
(Santiago et al., 2010).
200
Copyright © 2016 Dermatology Nurses' Association. Unauthorized reproduction of this article is prohibited.
Etiology
Common drug triggers include antiepileptics (the predom-
inant cause of DRESS), allopurinol, sulfonamides, and
antibiotic metabolites (Chiou et al., 2008; Choudhary
et al., 2013; Eshki et al., 2009). Although the exact etiology
is unknown, DRESS is most likely multifactorial. There is
a genetic component characterized by a deficit in enzymes
that break down drug metabolites (Choudhary et al., 2013).
In addition, there are probably associations with certain
HLA subtypes, such as has been described with HLA-B*1052
and carbamazepine-induced SJS (Chung et al., 2004).
Finally, certain drugs might trigger reactivation of a virus,
such as has been observed for the EpsteinYBarr virus (Kano
& Shiohara, 2004).
Management
Treatment of DRESS involves cessation of the offending
agent and supportive measures. Systemic corticosteroids
are often used although the data are insufficient (Zuliani,
Zwahlen, Gilliet, & Marone, 2005). There are successful
reports involving immunosuppressive agents such as cyclo-
phosphamide and cyclosporine (Laban et al., 2010; Zuliani
et al., 2005).
Visceral involvement can lead to pneumonitis, hepatitis,
renal failure, colitis, encephalitis, myocarditis, pericardi-
tis, cardiac failure, and pancytopenia, causing multiorgan
failure (Choudhary et al., 2013; Eshki et al., 2009).
Although most patients recover after cessation of the caus-
ative agent, the mortality rate is often reported at 10%
(Chiou et al., 2008; Eshki et al., 2009).
CONCLUSION
Although dermatology may have fewer emergencies com-
pared with other specialties, the mortality rates of those
diseases can be profound. In addition, it can be challeng-
ing to pinpoint the diagnosis. Many of the diagnoses are
made on clinical grounds without the benefit of standardized
diagnostic criteria. Several of the conditions described above
can present with a variety of different symptoms. Further-
more, the clinical presentations can change based on disease
progress. Finally, patients can have comorbid conditions
that exacerbate or mask their clinical presentation.
Although there are many other examples of diseases of
the skin that require immediate attention, we have at-
tempted to describe some of the more common states for
practitioners to be aware of. As with most conditions,
early intervention and diagnostic acumen are important for
long-term success in managing these patients. h
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